摘要
目的 探讨血栓性血小板减少性紫癜(TTP)患者的临床特征及预后影响因素.方法 回顾性病例系列研究.回顾性分析2013年1月至2022年12月宁夏回族自治区人民医院收治的15例TTP患者的临床资料,分析患者临床表现、实验室指标变化及预后影响因素.结果 15例TTP患者年龄(61±4)岁,包括男性8例,女性7例.神经、肾脏、消化、血液、循环、呼吸、风湿免疫系统受累分别有15、12、12、11、8、7、3例,发热9例,出血5例.血小板计数(Plt)和血红蛋白降低最常见,分别有15、14例,其次为乳酸脱氢酶(LDH)、D-二聚体升高及破碎红细胞出现,均有13例,再次为总胆红素升高(12例)、间接胆红素升高(12例)、平均红细胞体积(MCV)降低(11例)、ADAMTS13活性明显降低(8例)、肌钙蛋白T升高(8例)和网织红细胞计数(Ret)升高(5例).预后不良组(8例)的总胆红素、间接胆红素、D-二聚体、LDH和肌酐均高于预后良好组(7例)(均P＜0.05),Plt低于预后良好组(P=0.014).结论 TTP临床表现多样,可累及多个系统,Plt、血红蛋白、MCV、ADAMTS13活性降低,LDH、D-二聚体、总胆红素、间接胆红素、肌钙蛋白T、Ret升高及破碎红细胞出现均有助于TTP的诊断.Plt降低及总胆红素、间接胆红素、LDH、D-二聚体和肌酐升高可能为TTP患者预后不良因素.
Abstract
Objective To explore the clinical characteristics and prognostic influencing factors of patients with thrombotic thrombocytopenic purpura(TTP).Methods A retrospective case series study was conducted.The clinical data of 15 TTP patients who were admitted to the People's Hospital of Ningxia Hui Autonomous Region from January 2013 to December 2022 were retrospectively analyzed,and the clinical manifestations,changes in laboratory indexes and prognostic influencing factors of patients were analyzed.Results The 15 TTP patients were aged(61±4)years old,including 8 males and 7 females.The involvement of neurological,renal,gastrointestinal,hematologic,circulatory,respiratory,and rheumatologic-immune systems was found in 15,12,12,11,8,7,and 3 cases,respectively,with 9 cases of fever and 5 cases of bleeding.Decreased platelet count(Plt)and hemoglobin were most commonly present,with 15 and 14 cases,respectively,followed by elevated lactate dehydrogenase(LDH)and D-dimer,and the presence of fragmented erythrocytes,all in 13 cases,and then elevated total bilirubin(12 cases),elevated indirect bilirubin(12 cases),decreased mean corpuscular volume(MCV)(11 cases),significantly decreased ADAMTS13 activity(8 cases),elevated troponin T(8 cases)and elevated reticulocyte count(Ret)(5 cases)were also commonly detected.Total bilirubin,indirect bilirubin,D-dimer,LDH and creatinine in the poor prognosis group(8 cases)were higher than those in the good prognosis group(7 cases)(all P＜0.05),and Plt was lower than that in the good prognosis group(P=0.014).Conclusions The clinical manifestations of TTP are diverse and can involve multiple systems.Decreased Plt,hemoglobin,MCV and ADAMTS13 activity,and elevated LDH,D-dimer,total bilirubin,indirect bilirubin,troponin T and Ret,and the presence of fragmented erythrocytes contribute to the diagnosis of TTP.Decreased Plt and elevated total bilirubin,indirect bilirubin,LDH,D-dimer and creatinine may be poor prognostic factors for TTP patients.
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